Rare genetic flaw made her parents' choice a crucial one
By PAUL NYHANP-I REPORTER
Hannah Leigh woke at 4 a.m. Wednesday, unable to sleep on her first day of kindergarten.
But her journey -- and that of her parents -- was longer and harder than most.
Hannah's parents spent months questioning teachers, touring public schools and lobbying the people who would help them decide where their daughter would start school. Hannah's genetic code is scrambled just enough to pose serious developmental and physical problems.
The earnest 5-year-old with shocking red hair acts like any prospective kindergartner, selling lemonade on the corner and hoarding Hello Kitty stickers. But symptoms -- insatiable hunger, tantrums and cognitive delays -- threatened to creep in later during grade school like invasive weeds.
Everyone had an opinion. Hannah's preschool teacher suggested a school that was too far away for the family. Her dad wanted Hannah with 7-year-old brother Henry at View Ridge Elementary. And her mother was drawn to a Montessori program near their Viewridge home.
Hours later on Wednesday morning, Hannah finally arrived at Classroom 102 in Bryant Elementary, after three years, five school tours and a process that sometimes consumed her mother, puzzled her father and finally left Hannah in a place they can only hope is best.
As the city's public schools opened Wednesday, more U.S. parents knew their children were disabled than in years past -- nearly twice as many young kids were identified as disabled last year than 15 years earlier, according to the University of California-San Francisco's Stephen Kaye. These parents live in an uncertain world, where they now know what's wrong but not always how their kids will respond to cutting-edge therapies.
The confusion makes basic steps of childhood, such as the first day of school, even harder.
By Wednesday, though, Hannah was ready, pestering her mother all morning about climbing the nearby hill to school.
"I need to do work," Hannah said as she left behind a playground of screaming grade-schoolers to find her teacher, half her body obscured by a bright and largely empty pink Hello Kitty backpack.
Hannah Leigh didn't cry when she was born. Instead, she whimpered, then fell silent, a quiet, floppy baby with slack arms and listless legs.
For nearly two years, Barb and Rob Rose-Leigh, an independent house designer and public school teacher, didn't know what Hannah's silence meant. They feared their undersized baby suffered brain damage that would reveal itself over time.
The doctors weren't much help until a summer day three years ago when their new pediatrician told them Hannah had a rare genetic flaw, known as Prader-Willi syndrome. The hallmark was insatiable hunger that can become so severe adults have eaten themselves to death.
After waiting 20 months for some idea -- any idea -- of what was wrong, the news was a relief.
Yet Barb worried because what she knew about the genetic defect wasn't good.
One doctor already warned her that those with this disorder had no redeeming personality traits. Barb's mind filled with visions of a hunger that one child described as piranhas in his stomach, tantrums, obesity and institutionalization.
"This is all I know when my child is asked to be tested for it," said Barb, now 42.
The twist is that like with so many childhood disorders, the knowledge of Prader-Willi is literally changing as the Rose-Leigh family copes with it, thanks to genetic mapping, medical breakthroughs and new drugs. There is no cure, but doctors, researchers and parents can lessen symptoms.
"I just don't think you can predict where it's (Hannah's case) going to go," said Dr. David Springer, Hannah's pediatrician.
Ten years ago, doctors didn't even have a genetic test for Hannah's strain of Prader-Willi, and began prescribing human growth hormone to treat it only in the last few years. Now, Hannah gets daily shots, rotating among her arms, belly and legs.
Like many disorders, children with Prader-Willi fall along a spectrum from milder to severe cases -- Hannah has a milder type -- and doctors are still answering the question of how new drugs, healthful food and lifestyle changes will alter the symptoms.
Today, Hannah betrays few glaring symptoms. A shy girl, Hannah places herself right next to her teacher during circle time, belying a tendency to fixate on one thing, a letter, a coloring page or her new pink backpack. And you must look closely at her pale skin to see baby fat that reveals a lack of muscle tone, another Prader-Willi hallmark.
"Twenty or thirty years ago (doctors) told parents to take their kids home. They are just going to die from overeating," said Dr. Suzanne Cassidy, who has spent 26 years treating the disorder. "We are able to be much more optimistic ... ."
Hunting for a school
Last Valentine's Day, Barb Rose-Leigh was almost out of time because Hannah's Seattle school choices were due in two weeks.
So, she sat in the Daniel Bagley Elementary teachers lounge and peppered the principal with questions, her voice, as usual, a few speeds faster than a typical harried parent with young kids.
Everything at the 100-year-old school intrigued her -- the aging gym, plans for an autism class and the Montessori program -- anything that could allow Hannah to enroll in a traditional school near their home.
Bagley became Hannah's first choice, the fourth of five schools that Barb visited.
Like many parents of disabled children, Barb took a self-taught crash course in special-needs education. She studied the genetics of hunger, attended a law-school seminar, quizzed parents and educators, Googled and read anything she could find.
"There is help for you, but you have to many times step up and uncover that help," said Judy Winter, author of "Breakthrough Parenting for Children with Special Needs."
After the school tours, Barb sometimes retreated to the chaos of her home office, surrounded by piles of professional drawings, bills, her own children's artwork and a copy of "The Dalai Lama: The Path to Tranquility."
Since Hannah's symptoms could worsen, it was a complicated choice. The family had to balance her needs with the rest of the family, Henry and husband Rob, as mellow as Barb was intense.
Until recently, they all slept in one bedroom amid piles of books and magazines.
Barb pushed for a school closer to home, rejecting a suggestion that Hannah travel 30 minutes by bus to Broadview-Thomson Elementary in northwest Seattle.
"To take care of Hannah and my life I had to be healthy and that would (push) me over the edge," Barb said.
Prader-Willi families are fragile bodies. Parents are more likely to divorce and siblings more likely to carry emotional baggage, according to the Prader-Willi Association of America.
When choosing a kindergarten, these parents can think they cannot make a mistake. A mainstream student can bounce back from a bad school year, but parents worry a disabled child can't.
"Every year counts more, " says Lauren Roth of Lake Forest Park, whose 7-year-old daughter, Emma, has Prader-Willi syndrome. "She can't afford to get behind."
As Barb hunted for a kindergarten, her view of Hannah continued to evolve.
"I see Hannah continuing to wake up singing in 10 years. I see her wanting friendships that will become harder to attain and a majority of her self-esteem contingent on those relationships," Barb said.
By Feb. 28, Barb and Rob were done, and their choices -- Bagley first, Bryant Elementary second and Broadview-Thompson Elementary third -- were in the mail.
A month later, when Barb plucked the official response from the mailbox she didn't see any of those names.
Instead, Hannah appeared headed to John Rogers Elementary, a 300-student North Seattle school that Barb knew little about, though Hannah was tantalizingly close to her first choice, sitting second on Bagley's waiting list.
"I didn't sleep last night" after receiving Hannah's assignment, Barb said in early April as she hunted for breakfast in her cupboards, which -- like so many rooms in the house she designed -- have no doors, in part because Barb wanted it to feel open.
Hannah, however, was unconcerned as she carried her placement letter around the living room, dining room and kitchen, interested only in her name and her brother's.
The letter meant Barb packed Hannah into her VW Cabriolet on May 1 for a fifth school tour, this time of John Rogers. There she learned about one more option: an inclusion program.
"It seemed perfect," Barb recalled.
As the family warmed to this new idea they faced one more twist.
Earlier in the spring, Barb heard through the grapevine that Hannah had a spot at her second choice, Bryant Elementary in the Ravenna neighborhood.
Bryant was lost in the shuffle because the family's first choice was a general education class. That meant the school district placed Hannah in the first available general school, John Rogers Elementary.
But if Hannah didn't get into Bagley's Montessori program, Barb and Rob wanted her in Bryant's special-education classroom. If they wanted it, the spot was still available.
On May 8, Barb and Rob agreed to put Hannah in Bryant's blended classroom. It wasn't Montessori, but a nearby compromise where their daughter would learn alongside 10 typical and eight special-needs students.
And Hannah, Henry and Barb could ride their bikes to school every day.
The couple also abandoned their hopes for Bagley, just as they earlier gave up the idea that Hannah and Henry could walk to the same school together this fall.
"Next year it is Bryant ... I made it final and can't look back," Barb wrote in an e-mail sent May 9.
Four months later, Hannah hunted for her classroom's four yellow balloons on a Bryant playground packed with kids and picture-snapping parents, She was a little overwhelmed.
The shortest kid in a class of 18, Hannah watched her classmates more than teacher Kelley Clevenger, her half-smile breaking out when they talked.
The last to gather around the name board and among the last in line for recess, Hannah talked a little more as the morning wore on.
It only gets harder
Despite the angst of the last few months, Barb knew the school search wasn't over as she watched Hannah collect flowers in their front yard one August morning. It was just beginning.
Bryant Elementary is only a one-year fix. Next year, the family must decide where Hannah will attend first-grade. Rob still hopes she will join her brother at View Ridge.
Even after they find Hannah's first-grade classroom, it will get more complicated.
Kindergarten may be Hannah's easiest grade because she remains a small, adorable girl, with an earnest and halting way of talking and without the consuming hunger, tantrums and mental challenges that Prader-Willi can spawn later.
Children like Hannah may thrive in traditional preschools and kindergartens, which can give parents false hope their kids will break the mold and hang with "typically developing" students, wrote Nancy Finegold in the July-August Prader-Willi Syndrome Association newsletter.
But their symptoms may worsen in first, second and third grades, and the parents' hopes can collapse, wrote Finegold, whose daughter has the syndrome.
Just like Hannah, Finegold's daughter started kindergarten in an integrated classroom. By second grade she was in a contained special-education classroom.
Hannah's parents aren't focusing that far ahead. Their daughter's outlook remains muddied by the hopeful impact of human growth hormone, an optimistic pediatrician who refuses to predict her course and their own hope.
"I think she can ... live a happy life and a healthy life," Barb said.
"I do because I think things change."
ABOUT THE DISORDER
Prader-Willi syndrome strikes one in roughly 15,000 people at conception, a flaw created when genes are missing on a chromosome.
The syndrome's most distinctive feature is insatiable hunger that can become so severe caregivers must lock cupboards and even garbage cans. The condition is made even worse because those afflicted typically burn only about 1,000 calories a day, far less than the average person.
The combination of hunger and slow metabolism can lead to obesity, diabetes and death. The syndrome generates a host of other symptoms, including poor muscle tone, behavior problems, delayed motor development, learning disabilities and IQs typically in the 60s.
But the illness isn't necessarily debilitating. Babies with Prader-Willi have grown up to live in group homes, on their own, and some eventually attended college, with support.
There are more cases these days, not because the syndrome is more common, but because doctors have better diagnostic tools.
Today, some doctors and parents have higher hopes for treatment as well. Patients take human growth hormone, and new drugs and therapies that allow doctors to lessen some symptoms.
"We are able to be much more optimistic because we do have a number of therapeutic options -- (symptoms) can be controlled and (we) can have a much better outcome," said Dr. Suzanne Cassidy, a national expert on the syndrome who runs a Prader-Willi clinic in California.
-- Paul Nyhan
TO LEARN MORE
About Prader-Willi syndrome check the Prader-Willi Syndrome Association, www.pwsausa.org or 800-926-4797, and the Foundation for Prader-Willi Research, www.pwsresearch.org or 502-384-8405.P-I reporter Paul Nyhan can be reached at 206-448-8145 or firstname.lastname@example.org.